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<< Sickle Cell Disease And Other Hemoglobinopathies: Approaches To Emergency Diagnosis And Treatment

Epidemiology

The life expectancy for patients with SCD has significantly improved since the 1970s, when the median survival age was only 14 years.16 Today, most people homozygous for the HbS gene live to their mid-40s. Still, children still experience a large proportion of the morbidity and mortality related to SCD.

In the largest epidemiologic study of SCD (the Cooperative Study of Sickle Cell Disease), researchers enrolled a total of 2824 patients younger than  20 years. They recorded 73 deaths, mostly in patients with hemoglobin SS. The peak incidence of death was between 1 and 3 years of age, and infection was the major cause of mortality. After age 10, cerebrovascular accidents and traumatic events caused more deaths than did infection.17

In another study of nearly 300 children with SCA, the authors examined the frequency of the main clinical events over several years. In this study, 172 patients (58%) experienced one or more painful sickle cell crises. Over 40% suffered acute chest syndrome, while the prevalence of meningitis/ septicemia and osteomyelitis was 11.4% and 12%, respectively. Twenty patients (6.7%) developed stroke (peak prevalence at 10-15 years of age). Half of the patients had an episode of acute anemia due to either acute aplastic anemia or splenic sequestration.18

A larger study examined 694 children with sickle hemoglobinopathies over a 10-year period.19 Painful crises and ACS were the most common sickle cell-related events. Bacteremia occurred most frequently in SS children under 4 years of age and in SC patients less than 2 years of age. Twenty children, all with HbSS, died (mortality, 1.1 deaths per 100 person-years). Infection (mostly from Streptococcus pneumoniae and Haemophilus influenzae) was responsible for 11 deaths. Two children died of splenic sequestration, one died of cerebrovascular accident, and six died of undetermined causes.19

It may be possible to predict at an early age which children are most likely to experience complications of SCD. In one cohort study, researchers found three characteristics identified before age 2 that predicted later adverse events. These included an episode of dactylitis before the age of 1 year, a hemoglobin level of less than 7 g/dL, and leukocytosis in the absence of infection.20

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Last Modified: 07/23/2017
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